Purpose :
A mouse model systemically deficient in the ferroxidases ceruloplasmin (Cp) and hephaestin (Heph) exhibits retinal iron overload similar to that seen in humans with aceruloplasminemia. We tested the hypothesis that retinal ceruloplasmin production in this mouse model can protect against retinal iron accumulation.

Conclusions :
Instead of rescuing the DKO phenotype, intravitreal AAV-Cp injections worsened retinal degeneration as seen in fundus photographs and flat mounts. This degree of degeneration asymmetry between the experimental and control eyes has never been observed in uninjected DKO eyes (n>50). Moreover, lower Tfrc mRNA levels in the NSR of AAV-Cp WT mice suggest that the injections paradoxically caused retinal iron overload. The lack of differences in Tfrc mRNA levels in WT RPE suggests that intravitreal Cp delivery did not cause iron overload in the RPE in the short term. These results suggest that Cp in the neural retina may promote iron influx across the blood-retinal barrier.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.